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Common critical congenital heart defects and their association with cyanosis and dependence upon the ductus arteriosus

Common critical congenital heart defects and their association with cyanosis and dependence upon the ductus arteriosus
  Cyanosis? Ductal-dependent?
Left-sided obstructive lesions
Hypoplastic left heart syndrome Yes Yes
Valvar AS
  • Critical AS
Cyanosis or differential cyanosis* Yes
  • Moderate to severe AS
No No
COA
  • Critical COA
Differential cyanosis* Yes
  • Moderate to severe COA
No No
Interrupted aortic arch Differential cyanosis* (pattern of cyanosis varies based upon type) Yes
Right-sided obstructive lesions
Tetralogy of Fallot Variable Possibly
Tetralogy of Fallot with pulmonary atresia Yes Yes (unless multiple or large aortopulmonary collaterals are present)
Pulmonary atresia with intact interventricular septum Yes Yes
PS
  • Critical PS
Yes Yes
  • Severe PS
No No
Tricuspid atresia Yes Possibly
Severe neonatal Ebstein anomaly Yes PossiblyΔ
Parallel circulations
Transposition of the great arteries Yes Yes
Other
TAPVC Yes No§
Large VSD No No
AV canal defect No No
Truncus arteriosus Yes No
This table summarizes the key features of some of the more common critical CHD lesions. Critical CHD refers to lesions requiring surgery or catheter-based intervention in the first year of life. The most common lesions are listed in this table; however, there are other less common congenital heart lesions that may require intervention within the first year of life.
AS: aortic stenosis; COA: coarctation of the aorta; PS: pulmonic stenosis; TAPVC: total anomalous pulmonary venous connection; VSD: ventricular septal defect; AV: atrioventricular; CHD: congenital heart disease; RV: right ventricle; PDA: patent ductus arteriosus.

* In these lesions, the upper one-half of the body (preductal) is pink and the lower one-half (postductal) is cyanotic.
¶ Infants with tetralogy of Fallot or tricuspid atresia may have ductal-dependent circulation if there is severe RV outflow tract obstruction (ie, critical pulmonary stenosis or atresia).
Δ In cases of severe Ebstein anomaly with extreme cyanosis, a PDA may be necessary to maintain pulmonary blood flow until pulmonary vascular resistance drops.
◊ Reversed differential cyanosis (ie, oxygen saturation higher in the lower than upper extremity) may occur if there is coexisting coarctation of the aorta or pulmonary artery hypertension.
§ Some patients with obstructed TAPVC may require a PDA to maintain systemic cardiac output. However, a PDA may also increase the degree of cyanosis.
Graphic 103087 Version 5.0

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